Gustafson L
Department of Psychogeriatrics, University Hospital, Lund, Sweden.
Arch Gerontol Geriatr. 1987 Sep;6(3):209-23. doi: 10.1016/0167-4943(87)90022-7.
In a longitudinal prospective study of dementia, 158 patients were investigated post mortem. Sixteen patients were classified as frontal lobe dementia (FLD) of non-Alzheimer type and four cases as Pick's disease. Positive heredity for dementia was reported in 50% of these cases compared to 30% in a reference group of patients with Alzheimer's disease (AD). The typical clinical picture in FLD and Pick's disease was that of a slowly progressive dementia, at an early stage dominated by personality change, lack of insight, disinhibition, and later on stereotypy and increased apathy. There was also a progressive dynamic aphasia ending in mutism and amimia. Memory and spatial functions were comparatively spared. Disinhibition, oral/dietary hyperactivity, and echolalia were more consistently found in Pick's disease compared to FLD. The differential diagnosis against AD, cerebrovascular dementia, and other degenerative dementias and against affective disorders and psychotic reactions are discussed.
在一项关于痴呆症的纵向前瞻性研究中，对158名患者进行了尸检。16名患者被归类为非阿尔茨海默型额叶痴呆（FLD），4例为匹克氏病。这些病例中有50%报告有痴呆症的阳性遗传史，而阿尔茨海默病（AD）患者的参考组中这一比例为30%。FLD和匹克氏病的典型临床表现是缓慢进展的痴呆症，早期以人格改变、缺乏洞察力、脱抑制为主要特征，后期出现刻板行为和冷漠加剧。还存在进行性动态失语，最终导致缄默和模仿不能。记忆和空间功能相对保留。与FLD相比，脱抑制、口腔/饮食多动和模仿言语在匹克氏病中更常见。讨论了与AD、脑血管性痴呆和其他退行性痴呆以及情感障碍和精神反应的鉴别诊断。
